AB1793-HPR SPINAL INVOLVEMENT IN PAGET’S DISEASE OF BONE
Authors: A. Hriz, S. Ben Jemaa, A. Feki, M. Ezzeddine, M. H. Kallel, H. Fourati, R. Akrout, S. Baklouti
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Paget's disease of bone (PDB) is a benign acquired osteodystrophy characterized by excessive and disorderly bone remodeling that may affect one or more bones. The spine is the second site affected by Paget's disease (53%) after the pelvis (70%). Vertebral involvement is therefore likely to lead to complications which are fortunately relatively rare. <h3>Objectives</h3> to describe the clinical, biological and radiological aspects of the of PDB with spinal involvement, to highlight certain complications and to describe the management of patients with spinal localization. <h3>Methods</h3> This is a retrospective and descriptive study of the files of patients hospitalized for Paget's disease of bone with spinal involvement over a period of 20 years (Year 2000- Year 2020). <h3>Results</h3> Twenty-six patients (12 women and 14 men) were included. The average age of our patients was 66,8 years. The discovery of the PDB was fortuitous on standard radiographs and an elevation of PAL in 7,6% of cases. PDB was symptomatic in 92,4% of cases. Spinal pain was mechanical in 95,2% of cases. Three of our patients had spinal cord compression: Two patients had cervical compression and one patient had lumbar compression with Brown Sequard syndrome. Phosphocalcium balance was normal according to laboratory standards in all cases. An increase in PAL was found in 73% of cases. Standard X-ray of the dorsal spine showed condensation with widening affecting the vertebral bodies of D4, D8 and D11 in 43% of cases. Vertebral compression at levels D4, D8, D10 and D11 was found in 28.5% of cases. The radiograph of the lumbar spine showed condensation with hypertrophy of the vertebrae L1, L2, L3, L4, L5 in 37.5% of cases. A vertebral compression of L1, L2 and L4 was found in 31.25% of cases and an aspect of ivory vertebra in L3 and L4 in 12.5% of cases. A fibrillar aspect of the vertebral bodies of L1, L2 and L3 was objectified in 18.75% of cases. An aspect of vertebra in frame of L2 was observed in 6.25% of cases. Hypertrophy of the posterior L4 joints was reported in 6.25% of cases. Technetium 99m bone scintigraphy showed hyperfixation of a single seat in 9% of cases and multiple in 91% of cases. It had highlighted asymptomatic localizations in 9% of cases. Spinal CT was performed when there was doubt about the metastatic origin of the bone lesions in 5 cases, when there was low back pain or lumbo-radiculalgia in 5 cases. It showed signs in favor of PDB with corticomedullary dedifferentiation type in 6 cases, lytic bone lesions in 1 case and condensing lesions in 1 case, hypertrophy of the cortex and vertebral bodies in 1 case and vertebral collapse in 1 case. Spinal magnetic resonance imaging (MRI) was performed in 7 patients (27% of cases) when in doubt about the secondary metastatic origin of bone lesions in 3 cases (42.8%) or in search of a spinal cord compression in 4 cases (58.2%). It showed hypertrophy of the vertebral bodies, vertebral compression with recoil of the posterior wall in 4 cases and intramedullary hypersignal in 1 case. Sensorineural complications such as sensorineural hearing loss and occipital headache in 1 of our patients. Sarcomatous degeneration was observed in 1 of our patients. Drug treatment based on calcitonin or bisphosnate was administered in 77% of patients. The general evolution was good in 77.3% of cases (17 cases) with disappearance of pain in 95.3% of cases and normalization or maintenance of a normal level of ALP in 71.5% of cases. <h3>Conclusion</h3> Paget's disease of bone with spinal involvement is a serious disease that can compromise the functional and sometimes life-threatening prognosis of our patients. Diagnosis, early management and close monitoring of our patients are necessary to prevent its complications. <h3>References</h3> [1] Langston AL, Ralston SH. Management of Paget's disease of bone. Rheumatology (Oxford) 2004; 43 8: 955–959 [2] Altman RD, Brown M, Gargano F. Low back pain in Paget's disease of bone. Clin OrthopRelat Res 1987; 217: 152–161. [3] Hadjipavlou AG, Gaitanis IN, Katonis PG, Lander P. Paget's disease of the spine and its management. Vol. 10, European Spine Journal. 2001; p: 370–84. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.